Ann Thorac Surg 2009;88:2034-2035. doi:10.1016/j.athoracsur.2009.04.079
© 2009 The Society of Thoracic Surgeons
Case Reports
Rare Anatomic Location of a Papillary Fibroelastoma
Rubie Maybury, MDb,
Philip Mullenix, MDb,
Michael Greenberg, MDa,
Minling Liu, MDc,
Gregory Trachiotis, MDb,*
a Division of Cardiology, Veterans Affairs Medical Center, Washington, DC
b Division of Cardiothoracic Surgery, Veterans Affairs Medical Center, Washington, DC
c Division of Pathology, Veterans Affairs Medical Center, Washington, DC
Accepted for publication April 15, 2009.
* Address correspondence to Dr Trachiotis, Division of Cardiothoracic Surgery, Veterans Affairs Medical Center (VAMC)-VISN 5, VAMC/The George Washington University, 50 Irving St, NW Sec 112, Washington, DC 20422 (Email: gtrachiotis{at}mfa.gwu.edu).
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Abstract
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A rare anatomic location of a fibroelastoma arising form the edge of the right atrial wall near the atrioventricular node and the base of the septal leaflet of the tricuspid valve is presented. Complete surgical excision remains the goal with a precise and careful technique to ensure conduction integrity.
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Introduction
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Papillary fibroelastomas are tumors that typically occur on cardiac valves or adjacent endocardium. Although these tumors are benign and are generally asymptomatic, they are found more frequently because of broader applications of echocardiography. We present a rare case of a papillary fibroelastoma arising from both the tricuspid valve and the right atrium.
A 74-year-old man presented after a near-fainting episode. A stress electrocardiogram performed prior to this presentation for a chest pain syndrome had demonstrated no ischemic changes. A contrast chest computed tomographic scan at that time showed a mildly dilated ascending aorta and no evidence of pulmonary embolus. The patient was referred to our center and underwent a transthoracic echocardiogram, which confirmed mild dilatation of the ascending aorta and also demonstrated a 1 x 1 cm, ovoid right atrial mass attached to the septal leaflet of the tricuspid valve (Fig 1A). The patient underwent a coronary angiogram that showed normal coronary arteries and had a minor stroke after cardiac catheterization. Neurologic imaging was negative, and a repeat transthoracic echocardiogram and transesophageal echocardiogram showed no change in the right artial mass, and operative intervention was deferred for 6 weeks, at which time the patient had full recovery. The operation was conducted with transesophageal echocardiogram, bi-caval cannulation, use of a miniaturized cardiopulmonary bypass circuit under cardioplegic arrest, and tepid conditions with minimal cardiac manipulation. The choice to conduct this operation on an arrested heart was to avoid potential dislodgement of the tumor and to provide accurate and precise removal, because imaging studies suggested that the location of this tumor was near or at the atrioventricular nodal conduction system. The right atrium was entered, and a cardiac tumor was noted arising from the right atrial wall and extending across the zone of the atrioventricular node onto the septal leaflet of the tricuspid valve. The tumor was completely excised at the level of the endothelium, incorporating a fine ridge of endothelial margin along the conduction system, and a small piece of the septal leaflet with minimal atrial wall resection, avoiding the conducting system. The septal leaflet was repaired primarily with a single pledget of 3.0-polypropylene suture. The patient was discharged on postoperative day 5, in sinus rhythm, and with a normal echocardiogram.
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Fig 1. (A) Transthoracic echocardiogram illustrating right atria mass adjacent to septal leaflet of tricuspid valve. (B) Intraoperative photograph of resected fibroelastoma.
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On gross pathologic examination, the surgical specimen was a 1.2 x 0.8-cm, yellow lobulated mass demonstrating a grossly clean margin (Fig 1B). Histologic examination demonstrated papillary fronds with central hypocellular, hyalinized stroma covered by a single layer of endothelial cells. The stroma did not stain for muscle specific antigen or calretinin, ruling out atrial myxoma as best determined at permanent histologic analysis. These findings confirmed the diagnosis of papillary fibroelastoma.
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Comment
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Fibroelastomas occur sporadically [1, 2]. Although fibroelastomas make up only approximately 17% of all benign cardiac tumors, fibroelastomas are the most common cardiac valvular neoplasms [3], with 75% of fibroelastomas arising from the valvular endothelium [3]. Valvular cardiac papillary fibroelastomas arise almost exclusively in the mid-portion of the valve without encroaching on the annulus or free edge of the valve leaflet [4]. On the other hand, cardiac myxomas occur most commonly in the left atrium; in addition, myxomas are the most common right atrial intracavitary neoplasms [3]. Right atrial cardiac papillary fibroelastomas are exceedingly rare, with only 17 documented cases in the literature [2, 4, 5] (Table 1). Because most reported cases are symptomatic and treated surgically, their natural history is poorly defined [1, 2]. Based on the few reported cases discovered incidentally on echocardiogram and followed serially, fibroelastomas are believed to be slow-growing, although occasionally thrombi can form quickly on an existing fibroelastoma [2]. There are no randomized, controlled data to assess treatment options, and recommendations are based on expert opinion [2]. Symptomatic patients should undergo surgical excision, and symptomatic patients who are not surgical candidates should receive long-term oral anticoagulation [2]. Asymptomatic patients with pedunculated lesions who are surgical candidates should undergo resection; other asymptomatic patients may undergo surveillance with routine echocardiography [2], yet our opinion is for resection when diagnosed to prevent the potential lethal complications of cerebral emboli and stroke, obstruction to intracardiac blood flow, and coronary ostial occlusion. Surgical outcomes are generally excellent, and there are no reports in the literature of tumor recurrence after surgical excision [2].
In our patient, a preoperative echocardiogram obtained to evaluate pre-syncope demonstrated a cardiac mass arising from the septal leaflet of the tricuspid valve, and did not demonstrate a patent foramen ovale, or identify obstruction of the tricuspid valve. At the time of surgery, however, the tumor was found to arise primarily from the right atrial wall, with a portion of the base extending across the infundibular line onto the septal leaflet of the tricuspid valve. We believe that there have been no previously reported cases of a cardiac papillary fibroelastoma originating jointly from the right atrial wall and the tricuspid valvular endothelium.
In conclusion, right atrial papillary fibroelastoma is an extremely rare lesion, and when discovered in operative candidates, it should be resected. This is the first report of such a tumor involving both the right atrial wall and tricuspid valve.
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References
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- Libby P, Bonow RO, Mann DL, Zipes DP. Braunwald's heart disease: a textbook of cardiovascular medicine8th ed.. Saunders Elsevier: Philadelphia, PA; 2008.
- Gowda RM, Khan IA, Nair CK, Mehta NJ, Vasavada BC, Sacchi TJ. Cardiac papillary fibroelastoma: a comprehensive analysis of 725 cases Am Heart J 2003;146:404-411.[Medline]
- Burke A, Jeudy J, Virmani R. Cardiac tumors: an update Heart 2008;94:117-123.[Free Full Text]
- Gabbieri D, Rossi G, Barutti L, Corghi F, Zaca F, Sarandria D, et al. Papillary fibroelastoma of the right atrium as an unusual source of recurrent pulmonary embolism J Cardiovasc Med 2006;7:373-378.
- Lotto AA, Earl UM, Owens WA. Right atrial mass: thrombus, myxoma, or cardiac papillary fibroelastoma? J Thorac Cardiovasc Surg 2006;132:159-160.[Free Full Text]
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Abstract
Introduction
